Management of severe acute chest syndrome in a patient with a history of severe delayed haemolytic transfusion reaction.

A case of a patient in her 30s with sickle cell anaemia who developed multiorgan failure and Acute Respiratory Distress Syndrome(ARDS) from acute chest syndrome (ACS) despite a simple red blood cell transfusion is presented in this report. She was treated with plasma exchange (PLEX) due to a history of severe delayed haemolytic transfusion reaction and a lack of available compatible units of packed red blood cells. Following one session of PLEX with half plasma/half albumin, she had rapid clinical improvement from mechanical ventilation to room air in 5 days. This finding is consistent with existing case reports of rapid improvement in pain, oxygenation and organ function following PLEX. Proposed mechanisms include the modulation of rheological properties of red blood cells by plasma, as well as the removal of the cytotoxic effects of haptoglobin and haemopexin. This case demonstrates the effectiveness and safety of an alternative upfront approach to managing complications related to ACS in those who are heavily alloimmunised.